What is pheochromocytoma?
Pheochromocytoma is a type of neuroendocrine tumor that grows from cells called chromaffin cells. These cells produce hormones needed for the body and are found in the adrenal glands. The adrenal glands are small organs located in the upper region of the abdomen on top of the kidneys. About 80-85% of pheochromocytomas grow in the inner layer of the adrenal gland, called the adrenal medulla. About 15-20% of pheochromocytomas grow outside of this area and are called extra-adrenal pheochromocytomas or paragangliomas.
Most pheochromocytomas are benign, which means they are not cancer and do not spread to other parts of the body. Only about 10% of pheochromocytomas spread to other parts of the body.
How common is pheochromocytoma?
It is unknown how many people have pheochromocytoma because many people are never diagnosed. Most cases of pheochromocytoma occur in people aged 30 to 50 years old. One estimate suggests about only 8 people per 1 million people have pheochromocytoma, but this estimate may be low.
How is pheochromocytoma diagnosed?
Some people with pheochromocytoma have symptoms, but others don’t. Symptoms may occur as often as several times a day to a couple of times per month. Some people may feel intense symptoms that last for a short period of time, called “paroxysmal attacks”. These symptoms can include:
• High blood pressure
• Headaches
• Irregular heartbeat
• Sweating
Lab Tests: If you have symptoms of pheochromocytoma, your doctor will order lab tests of your urine and blood to check your hormone levels.
Imaging: Your doctor will use imaging scans such as CT, MRI, and PET to look at where the tumor is and how big it is. They will also check for signs that the tumor has spread to other parts of the body.
How is pheochromocytoma treated?
Treatment of pheochromocytoma may involve many different doctors, including doctors who specialize in hormone disorders and doctors who diagnose and treat cancer. Treatment options to discuss with your doctor include:
Medications: Your doctor may give you medications to control your symptoms, such as alpha blockers and beta blockers, which are drugs to control high blood pressure.
Surgery: Surgery is used to remove as much of the tumor as possible. In some cases, the entire adrenal gland may be removed.
Radiation therapy and chemotherapy: Radiation and chemotherapy treatments are used when pheochromocytoma has spread to other parts of the body.
Does pheochromocytoma run in families?
In some cases, pheochromocytoma can run in families. About 25-35% of cases of pheochromocytoma may be inherited. Some of these inherited cases may be associated with a genetic condition, such as Multiple Endocrine Neoplasia Types 2a and 2b, Von Hippel-Lindau Syndrome, and Neurofibromatosis.
How does pheochromocytoma form?
Scientists have found mutations in approximately 20 different genes that they think may lead to pheochromocytoma and paraganglioma. Mutations in the genes RET, VHL, NF1, SDHA, SDHB, SDHC, SDHD, SDHAF2, MDH2, IDH1, PHD1/PHD2, HIF2A/EPAS1/2, TMEM127, MAX, HRAS, MAML3 and CSDE1 may play a role in forming pheochromocytoma and paragangliomas. In many cases, it is not known what causes pheochromocytoma to form.
If you have pheochromocytoma, you may have other genetic conditions that increased your chance of getting pheochromocytoma. These genetic conditions include:
• Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B)
• Von Hippel-Lindau (VHL) syndrome
• Neurofibromatosis type 1 (NF1)
• Hereditary paraganglioma syndrome
• Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor [GIST])
• Carney triad (paraganglioma, GIST, and pulmonary chondroma)
What is the prognosis for someone with pheochromocytoma?
The estimate of how a disease will affect you long-term is called prognosis. Every person is different and prognosis will depend on many factors, such as:
• Where the tumor is in your body
• If the cancer has spread to other parts of your body
• How much of the tumor was taken out during surgery
If you want information on your prognosis, it is important to talk to your doctor. Also, NCI has resources to help you understand cancer prognosis.
Doctors estimate pheochromocytoma survival rates by how groups of people with pheochromocytoma have done in the past. Patients with a small pheochromocytoma that has not spread to other parts of the body have a five-year survival rate of about 95%. Patients with pheochromocytoma that has grown back (recurred) or spread to other parts of the body have a five-year survival rate between 34% and 60%.
FAQs
A pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare tumor that grows in an adrenal gland. Most often, the tumor is not cancer. When a tumor isn't cancer, it's called benign. You have two adrenal glands — one at the top of each kidney.
What are the signs and symptoms associated with pheochromocytoma? ›
What Are the Signs and Symptoms Associated with Pheochromocytoma?
- Headaches.
- Heart palpitations, marked by pounding, fluttering or beating rapidly.
- Hypertension (high blood pressure), though levels may rise and fall and sometimes even drop too low.
- Pale appearance.
- Sweating.
- Tremors, or shaking.
The 5-year relative survival rate for localized pheochromocytomas is 95%. The 5-year relative survival rate for a pheochromocytoma that has spread or recurred is estimated to be between 34% and 60%. Experts measure relative survival rate statistics for tumors and cancers every 5 years.
What is the best treatment for pheochromocytoma? ›
Ninety percent of patients are cured by surgery to remove benign pheochromocytoma tumors. Surgery for tumor removal is typically done by laparoscopy, during which a small incision is made in the abdomen.
Is pheochromocytoma life threatening? ›
Without treatment, pheochromocytomas can lead to life-threatening high blood pressure and complications including heart attack, heart arrhythmias, pulmonary edema, heart failure, and stoke, among others. With treatment, the outlook is better. In many cases, surgical removal of the tumor can cure people of the disease.
How does a pheochromocytoma make you feel? ›
With a pheochromocytoma, the tumor releases hormones that can cause various symptoms. They include high blood pressure, headache, sweating and symptoms of a panic attack. If a pheochromocytoma isn't treated, serious or life-threatening damage to other body systems can happen.
What are the 5 H's of pheochromocytoma? ›
Although hypertension, hypermetabolism, hyperglycemia, headache, and hyperhidrosis (the 5 H's) are usually the dom- inant clinical manifestations of pheochromocytoma, approxi- mately 30% of these patients do not present these signs (7).
What age do people get pheochromocytoma? ›
Pheochromocytoma can occur at any age. However, it is diagnosed most frequently between the ages of 30 and 50. Up to 20% of pheochromocytomas are diagnosed in children.
Where is the pain with a pheochromocytoma? ›
Other Typical Signs and Symptoms of Pheochrmocytoma Include:
Anxiety and nervousness. Nervous shaking (tremors) Pain in the lower chest or upper abdomen.
What happens if pheochromocytoma is left untreated? ›
If pheochromocytomas are left untreated, they can potentially cause serious, life-threatening complications, including: Heart muscle disease (cardiomyopathy). Inflammation of your heart muscle (myocarditis). Uncontrolled bleeding in your brain (cerebral hemorrhaging).
Thyrotoxicosis, hypoglycemia, anxiety or panic attacks, hyperthyroidism, adrenal medullary hyperplasia, familial dysautonomia, and intracranial lesions may also have similar symptoms. Various tumors including neuroblastomas, ganglioneuroblastomas and ganglioneuromas may mimic pheochromocytomas/paragangliomas.
What foods should be avoided with pheochromocytoma? ›
Foods rich in tyramine like red wine, chocolate, dried or smoked meats, and some cheeses should be avoided in pheochromocytoma. Other foods can increase catecholamines in the body, triggering and/or mimicking symptoms of pheochromocytoma.
What is the 10 rule for pheochromocytoma? ›
Previously, many physicians referred to pheochromocytoma as “the 10 percent” tumor, meaning that 10% of cases were familial (inherited), 10% bilateral (affecting both right and left adrenal glands), 10% malignant (demonstrating cancerous metastases to other sites in the body), 10% in children, and 10% lying outside of ...
What famous person has a pheochromocytoma? ›
Famous Cases
President Eisenhower- After his death, an autopsy revealed a 1.5-cm pheochromocytoma in the left adrenal gland. On analysis of his blood pressure through his life, fluctuating systolic and diastolic blood pressure spikes were documented.
Can you live a long life with pheochromocytoma? ›
Results: Radically operated (n=162, 81.8%) patients (median age 52.0 years (Q1–Q3: 39.3–63.0)) had an overall 5- and 10-year survival after surgery of 90.8% (CI95%: 84.5–94.6) and 78.7% (CI95%: 69.8–85.2) and a recurrence-free 5- and 10-year survival of 87.0% (CI95%: 80.1–91.7) and 75.7% (CI95%: 66.7–82.7), ...
How long do pheochromocytoma attacks last? ›
They can be short term or long lasting. Sudden attacks often last less than 1 hour. They can happen several times a week, or only a few times a year. Women experience these spikes more often than men.
What are the 2 most common diagnostic tests for pheochromocytoma? ›
M-iodobenzylguanidine (MIBG) imaging, a scan that can detect tiny amounts of an injected radioactive compound. The compound is taken up by pheochromocytomas. Positron emission tomography (PET), a scan that also can detect radioactive compounds taken up by a tumor.
What is the cardinal indication of a pheochromocytoma? ›
Cardinal symptoms include sevre headache (72 to 92 per cent), sweating (60 tp 70 per cent), palpitations (51 to 73 per cent), and hypertension (> 90 per cent) of which 50 per cent is sustained, 50 per cent paroxysmal.
When should you suspect pheochromocytoma? ›
Signs or symptoms of pheochromocytomas may include:
High blood pressure. Rapid or forceful heartbeat.
What is the rule of 10 for pheochromocytoma? ›
According to the "rule of 10", in 1/10 patients with pheochromocytoma it is malignant, in 1/10 of cases the tumor is bilateral, in 1/10 extra-adrenal and in 1/10 familial.
